Focal fibrocartilaginous dysplasia of long bones: report of eight additional cases and literature review.

Abstract

We report eight additional cases of focal fibrocartilaginous dysplasia (FFCD) in the proximal tibia (five), distal ulna (one), and distal femur (two). Spontaneous, complete resolution of the lesion was observed in two tibiae and one ulna. Three tibial lesions with genu varum deformity were managed with osteotomy. Two femoral FFCDs caused persistent or progressive deformity: one genu valgum with patellar dislocation, and one genu varum. These patients underwent concomitant deformity correction and lengthening by the Ilizarov method. The final results were satisfactory in all patients except one, who underwent valgus tibial osteotomy and developed mild postoperative genu valgum. The analysis of a total of 46 cases in the literature and our experience suggests that (a) FFCD has a wide histopathologic spectrum, ranging from purely dense, fibrous tendon-like tissue to benign fibrocartilaginous tissue; (b) at least 45% of tibial FFCD demonstrates progressive, spontaneous resolution; (c) in contrast, femoral and humeral FFCDs appear to have a slim possibility of spontaneous regression of the deformity; and (d) corrective osteotomy is indicated when the deformity is increasing or persistent, or when the existing deformity is severe enough to jeopardize adjacent joint mechanics and alignment.